Objective. To present a clinical case of surgical correction of a craniovertebral anomaly complicated after 8 years by distal junctional kyphosis, stenosis, antelisthesis of the C5 vertebra and compressive ischemic cervical myelopathy in the C5–C6 segment.Material and Methods. When treating a 56-year-old patient with multiple anomalies of the craniovertebral region, differentiated surgical technologies were consistently used due to the development of late complications. The sequence and rationale for surgical decision making is described.Results. Initially, the patient underwent transoral decompression and posterior occipitocervical fixation, and after 8 years - reinstallation of the system with distal extension of the instrumentation zone to the C7 vertebra with indirect posterior decompression of the spinal cord, anterior discectomy with direct decompression and cage fixation at the C5–C6 level. Regression of myelopathic syndrome, correction of orthopedic status and significant improvement in functional status were achieved.Conclusion. A rare clinical observation demonstrates a combination of basilar invagination with assimilation of the atlas, which has provided rationale for two-stage surgical treatment in one surgical session (1st stage – transoral resection of the dens and 2/3 of the C2 vertebral body with anterior decompression of the spinal cord, and 2nd stage – occipitocervical fixation). The use of extended systems in this case caused the development of a clinically significant syndrome of the distal adjacent level, which required repeated surgical treatment after 8 years.